DESCRIPTION: Since it discovery by modern medical science in the early part of this century, SCD has been very widely studied. It was the first disease to be described as a molecular disease as the difference between sickle hemoglobin (Hb S) and normal adult hemoglobin was elucidated. When the genetic code was discovered, the mutation responsible for the amino acid substitution in the beta globin of Hb S was also determined. The beta hemoglobin gene was discovered as far back as 1977. The processes involved in the pathophysiology of SCD have all been extensively researched. The clinical course of SCD has also been studied in large number of patients through well-organized multi- institutional studies such as the Cooperative Study of Sickle Cell Disease. In the United States and other technologically developed countries where SCD is prevalent, much progress has been made in its diagnosis, treatment, an comprehensive management. Today, it is expected that over 90% of children with SCD born in the United States will survive beyond 20 years of age. The latest mortality data on SCD patients in the United States show that half of the patients now survive beyond their mid 40's. A few patients have been cured through bone marrow transplantation in Europe and the United States. However, in all of the United States only about 1,000 babies are born each year with SCD and the entire country has an estimated 60,000 patients. The WHO estimates that 250,000 babies with SCD are born each year of which more than 150,000 are in Africa. In Central and West Africa with Hb S prevalence of 15-30%, approximately 2% of all babies have a form of SCD. The vast majority of them go undetected and will probably die of complications of the disease before anyone realizes they have SCD. No statistics of deaths directly or indirectly resulting from SCD are kept by African countries. The impact of SCD on public health is largely unknown to public health officials in most African countries. The fact that children with SCD do not die from SCD per se, but from malaria and other infections, from respiratory illnesses, and from diarrhea and dehydration makes it easy to lose track of those with SCD among the many children dying from these well recognized "major killers" of children. Buried within the grim statistics of childhood mortality in Africa due to these diseases are even worse data on mortality of children with SCD. Sickle cell disease in Africa is not fundamentally different from SCD in the United States. However, the clinical expression of the disease is vastly different because of the special influence of malaria. Malaria is thought to be the leading cause of death and morbidity in SCD patients in Africa. It is also thought to be the commonest precipitating cause of the painful episode. The contrasting relationship between malaria and sickle cell trait on one hand and malaria and SCD on the other has not been fully explored. While penicillin prophylaxis to prevent pneumococcal septicemia is the major preventive therapy for children with SCD in the United States, anti-malarial prophylaxis may be the most important preventive therapy for those in Africa. Unfortunately, the rapid rate of development of resistance by plasmodium species to anti-malarial agents have made anti-malaria prophylaxis quite complex. One of the major and often life-saving therapeutic modalities employed in the management of SCD is blood transfusion. Among the many risks of blood transfusion are those due to transmitted infectious agents. Various forms of hepatitis, malaria, bacteria, and HIV have all been transmitted through the transfusion of blood products. The prevalence rates of these infectious agents in Africa is quite high. To what extent are SCD patients in African countries endangered by blood transfusion? Transfusion practices in many African countries may not be at the level of safety practiced in technologically advanced countries. Should the indication for blood transfusion of SCD patients be different in African countries because of the danger of posttransfusion infections?